Joint hypermobility and its relationship to musculoskeletal pain in schoolchildren: a cross-sectional study.

OBJECTIVES: To determine if joint hypermobility is associated with musculoskeletal pain in a population of Italian schoolchildren. DESIGN: Cross-sectional, school-based study, using a pretested questionnaire administered to schoolchildren to enquire about musculoskeletal pain and Beighton criteria, with score of > or =5 as a cut-off, to test for hypermobility. SETTING: Eight primary schools in the town of Cesena, Italy. PARTICIPANTS: 1230 Italian schoolchildren aged 7 to 15 years representing an opportunistic sample of 10% of the schoolchildren in Cesena MAIN OUTCOME MEASURES: (1) The strength of association between hypermobiliy and musculoskeletal pain; (2) the impact of hypermobility on daily activities, using a subjective "disability score" and a "physical activity score." ANALYSIS: Sample size calculation for evaluating if hypermobility was associated with musculoskeletal pain was performed prior starting the study. Children experiencing pain at least once a week were used as cases, children experiencing pain seldom or never served as controls. RESULTS: A total of 1046 consenting Italian schoolchildren (mean age 10.8 years) were included. The prevalence of musculoskeletal pain reported by schoolchildren was 18%. 22% of children with musculoskeletal pain versus 23% of controls had hypermobility (OR 1.057, 95% CI 0.7 to 1.4). Functional limitations measured by a "disability score" correlated in a weak negative way with Beighton score (p = 0.03). The "physical activity score" correlated in a weak positive way with Beighton score (p = 0.012). CONCLUSIONS: No association was found between hypermobility and musculoskeletal pain. Hypermobile children did not experience functional limitations in daily activities, and they were slightly more active than non-hypermobile children.

[Periodic fever, aphthous stomatitis, pharyngitis, and lymphadenopathy: a pediatric caseload]. / Febbre periodica, stomatite aftosa, faringite, linfoadenopatia (PFAPA): una casistica pediatrica.

We report 5 patients (3F; 2M; age 19-60 months) affected by a syndrome characterized by recurrent episodes of abrupt onset of fever, aphtous stomatitis, pharyngitis and cervical adenopathy named by the acronym PFAPA (periodic fever, aphtous stomatitis, pharyngitis and adenopathy). The episodes had clockwork periodicity (3-4 weeks) and the fever was unaccompanied by remarkable respiratory signs or symptoms. All children were doing well between episodes. The inflammatory markers were elevated only during attacks in all children. Steroid therapy was highly effective in controlling symptoms in 5/5 patients and tonsillectomy was associated with remission in two of five patients. In one child the syndrome resolved spontaneously, in two patients resolved after tonsillectomy and in two children persisted. No long-term sequelae were observed.

[Kluver Bucy syndrome and central diabetes insipidus: two uncommon complications of herpes simplex encephalitis]. / Sindrome di Kluver Bucy e diabete insipido centrale: due rare complicanze dell'encefalite erpetica.

Herpes Simplex Encephalitis (HSE) is an uncommon but severe disease with high mortality and morbidity. The major clinical manifestations are deteriorating consciousness with confusion, drowsiness or coma, altered behaviour, convulsions and a variety of neurological signs (hemiplegia, aphasia, ataxia, etc.). An uncommon complication of HSE is Kluver Bucy syndrome (KBS), characterized by hyperorality, bulimia and changes in emotional behaviour. Neuroimaging studies frequently show an involvement of the temporal lobes and limbic areas. Another uncommon complication of HSE is central diabetes insipidus as a result of herpes simplex infection of the hypothalamus. We report two pediatric cases of HSE complicated with Kluver Bucy syndrome and central diabetes insipidus.

[Retrospective analysis of Henoch-Schonlein purpura in children: diagnostic difficulties and renal outcome]. / Analisi di una casistica pediatrica di porpora di Schönlein-Henoch: difficoltà diagnostiche iniziali e follow-up a distanza.

OBJECTIVE: To assess clinical presentation, diagnostic difficulties, natural history and prognostic indicators of renal involvement of Henoch Schönlein Purpura, a ten-years retrospective analysis of all patients case records was performed. METHODS: After a variable interval of 1 to 10 years following the vasculitic episode all the children came to the outpatient clinic for history taking, clinical examination, blood pressure measurements, urine analysis and renal function assessment. RESULTS: 54 children, mean age 5.9 +/- 2.5, were identified. The purpuric rash was associated with arthritis in 46 patients (85%), abdominal pain in 28 (51.8%), temperature > 38 degrees C in 11 (20%). The purpuric rash was present at on presentation in 39 subjects (72%) whereas in 15 children (28%) it followed the subsequent signs or symptoms that were arthritis in 8 (53.3%), joint pain in 2 (13.3%), abdominal pain in 5 (30.3%). Abdominal pain was the only symptom of presentation in 5 patients; two of them underwent surgical laparotomy.

Undiagnosed coeliac disease and risk of autoimmune disorders in subjects with Type I diabetes mellitus.

AIMS/HYPOTHESIS: We tested the hypothesis that silent coeliac disease is more frequent than expected in both patients with Type I (insulin-dependent) diabetes mellitus and their first-degree relatives. We evaluated how the presence of other autoimmune disorders in diabetic patients and their first-degree relatives is related to silent, unrecognized coeliac disease. METHODS: Sera from 491 subjects with Type I diabetes, 824 relatives and 4,000 healthy control subjects were screened for anti-endomysial antibodies and all those subjects who tested positive for anti-endomysial antibodies underwent intestinal biopsy. RESULTS: We found that the prevalence of coeliac disease was 5.7 % among the diabetic patients and 1.9 % among the relatives, values significantly higher than those found among the control subjects (p < 0.0001; p < 0.001). The prevalence of autoimmune disorders in diabetic patients with coeliac disease was significantly higher than in subjects with Type I diabetes alone (p < 0.0001). The prevalence of autoimmune disorders in the relatives with coeliac disease was significantly higher than in those who tested negative for anti-endomysial antibodies (p = 0.01). CONCLUSION/INTERPRETATION: This report provides further confirmation of the high prevalence of undiagnosed coeliac disease among diabetic patients and their relatives. This interesting new finding is the increased presence of other autoimmune diseases in these patients, as well as in their relatives with a delayed diagnosis for coeliac disease. Patients newly diagnosed with coeliac disease showed excellent compliance with the gluten-free diet. This should encourage policymakers to consider introducing an easy-to-use screening programme for diabetic patients and their relatives into everyday clinical practice, in order to prevent coeliac-associated symptoms and the onset of additional, more serious auto-immune disorders.

[Etiology and precipitating factors of cyclic vomiting]. / Eziologia e fattori precipitanti del vomito ciclico.

The aim of the study was to determine the nature, severity, precipitants and associated features of attacks and the incidence of potential aetiological factors of cyclic vomiting syndrome (CVS). Ten patients (6 boys and 4 girls aged 2-12 years) with CVS, defined as recurrent episodes of vomiting with symptom-free intervals occurring two or more times per year, with episodes having a similar pattern and for which no other cause could be found, ten patients (7 boys and 3 girls aged 7-14 years) with migraine, defined as recurrent headache with symptom-free intervals and at least three of the following symptoms or associated findings: abdominal pain, nausea or vomiting, throbbing headache, unilateral location, associated aura (visual, sensory, motor), relief after sleeping and ten controls (6 boys and 4 girls aged 4-13 years) were studied. The mean age at onset of symptoms in patients with CVS was 3.9 years and the mean number of attacks per year was 4, the average duration of episodes was 20.5 hours and the mean number of school missed days were 6 per year. The mean age at onset of symptoms in patients with migraine was 7.6 years and the mean number of attacks per year was 9.9, the average duration of episodes was 8 hours and the mean number of school missed days were 6.4 per year. A family history of migraine was significantly higher in both groups of patients, compared with controls (p = 0.009). A personal history of headache and/or migraine and associated sign or symptoms like recurrent abdominal pains, limb pain and kinetosis was significantly higher in both patients compared with controls (p < 0.001). CVS is a chronic, disabling condition and is a migraine variant, with attacks usually precipitated by stress and intercurrent infections.

[Predictive factors of etiology (bacterial or viral) in acute gastroenteritis in childhood]. / Fattori predittivi dell'eziologia (batterica o virale) nelle gastroenteriti acute dell'infanzia.

The aim of the study was to identify immediate clinical and/or laboratory findings able to differentiate bacterial from viral etiology of acute gastroenteritis in pediatric patients. We studied 52 children, aged between 5 months and 12 years, consecutively admitted to hospital with acute diarrhoea lasting less than 5 days. All the patients were divided into 4 groups according to etiologic agent, subsequently demonstrated by culture: salmonellae (group A), rotavirus (group B); combined salmonellae and rotavirus (group C) and no pathogen (group D). The contemporary presence of fever > 39 degrees C, number of daily liquid stools > 6, presence of bloody diarrhoea, positivity of C-reactive protein and hyponatremia (< 135 mEq/l) allowed to recognize the etiology (viral or bacterial) before results of culture (sensitivity was 71% and specificity was 97%). In particular, hyponatremia resulted significantly lower in group A and C than in group B and D. We concluded that hyponatremia can be considered a marker for acute gastroenteritis caused by salmonellae.

[Evaluation of diosmectite in acute diarrhea in children]. / Valutazione della diosmectite nella diarrea acuta in età pediatrica.

The aim of this case-control study, was to estimate clinical effects, safety and parents' assessment of Diosmectite, in acute infantile diarrhoea. Thirty-five children aged 0-5 years, with acute diarrhoea from at least 5 days received alternatively oral rehydration solution (ORS) and Diosmectite (3 g/day for 3 days in children aged under 3 years and 6 g/day for 3 days in children aged over 3 years) (16 cases) or ORS only (19 cases). At discharge, parents' opinion concerning satisfaction for treatment received was evaluated through a questionnaire. Differences in the amount of liquid intake, weigh recovery, resolution of fever, resolution of vomiting and number of loose stools in the first 48 hours of hospitalization were not significant. Diosmectite was well tolerated and no major side effects were found. Parents' satisfaction for therapy resulted higher in the treated group than in the control group, even if differences were not significant. Treatment with ORS and Diosmectite allowed to shorten the mean duration of hospitalization by 0.4 days/children in the treatment group compared to the control group. Further studies with more cases and with higher does of the drug are necessary to confirm our results.

Transient focal neurologic deficits associated with hypoglycaemia in children with insulin-dependent diabetes mellitus. Italian Collaborative Paediatric Diabetologic Group.

We describe 54 transient focal neurologic deficits (TFND) episodes in 44 children under 18 y observed retrospectively during a 5-y period (1991-96). Mean age and duration of insulin-dependent diabetes mellitus (IDDM) were 8.4 and 3.4 y, respectively. None of the children had a history of seizure disorder and only one had a personal history of migraine. Twenty-nine episodes were characterized by right- and 25 by left-sided hemiparesis. Three of six patients who presented more than one event had alternate episodes of right- and left-sided hemiparesis. On 8 occasions the episode was preceded by a brief convulsion, in 39 it was not witnessed, and in 7 it was certainly absent. Hypoglycaemia (< or = 2.77 mmol/l) was documented on 26 occasions. On 18 of these 26 occasions, the episodes did not resolve promptly after sugar administration. The clinical course was benign, all patients remained neurologically normal and none developed migraine at follow up. Episodes of TFND were associated with hypoglycaemia in the majority of our cases and we do not consider invasive investigations to be mandatory, since the long-term prognosis was invariably good.

[Prevalence of Chlamydia pneumoniae in respiratory infections in children: an ambulatory diagnostic problem]. / Prevalenza di Chlamydia pneumoniae nelle infezioni respiratorie del bambino: un problema diagnostico ambulatoriale.

It has been recently suggested that Chlamydia Pneumoniae infection is a common finding among children with acute respiratory diseases. Chlamydia cell culture is difficult and time-consuming to perform. Polymerase chain reaction (PCR) is a more rapid but also more expensive technique used to identify Chlamydia in pharyngeal swab, but it can be performed only in few specialized laboratories. We tested a rapid enzyme immuno-assay to detect Chlamydia in 20 children with respiratory infections (mean age 3.29 years; male:female ratio = 12:8) and in 21 healthy children (mean age 4.70 years male:female ratio = 15:6). Prevalence of Chlamydia isolation from pharyngeal swab was very high in both patients and healthy children without a significative difference in the two considered groups (45% vs 42%, p = 0.8). Specific Chlamydia IgG antibodies were undetectable in all patients and healthy children. Nine out of 20 patients affected by acute respiratory disease were Chlamydia-positive and 11 out 20 were Chlamydia-negative: these two groups didn't differ in regard to clinical and laboratory features, whereas duration of symptoms was significantly longer in Chlamydia-positive patients (9.3 vs 5.5 days, p = 0.014). Our study suggests a high prevalence of Chlamydia pharyngeal swab positivity in both healthy and sick children. Diagnosis of Chlamydia infection was not feasible on the basis of the considered clinical and laboratory findings.

High risk of Helicobacter pylori infection associated with cow's milk antibodies in young diabetics.

Antibody titres (IgA and IgG) for Helicobacter pylori were assayed in 69 insulin-dependent diabetes mellitus patients (42 males, age 1-20 years) and 310 healthy controls (171 males, age 1-20 years). A positive antibody titre for Helicobacter pylori was found in 18/69 diabetic subjects compared to 17/310 controls (p < 0.001). There was no difference between Helicobacter pylori positive and negative diabetic subjects as regards age, sex, duration of diabetes, diabetic control, insulin dose and SDS for weight and height. Gastroduodenoscopy revealed presence of Helicobacter pylori and evidence of gastric inflammation in 7/8 symptomatic diabetic children. There was a significant association in the diabetic subjects between positivity for anti-cow's milk protein and anti-Helicobacter antibodies, compared to the control group. Seven of the 17 diabetics studied within 3 months of the onset of diabetes had positive antibody titres for Helicobacter. Of these seven patients, five were positive for anti-cow's milk protein antibodies. In our study the prevalence of Helicobacter pylori infection was significantly higher in diabetic subjects than in controls, but the infection was asymptomatic and there was no correlation with diabetes control. In diabetic subjects Helicobacter pylori infection was associated with a humoral response to cow's milk proteins and was often present from the onset of diabetes.

[Blood sugar control in children and young diabetics during an educational summer camp]. / Controllo glicemico in bambini e giovani diabetici durante un soggiorno educativo estivo.

UNLABELLED: The aim of the study was to evaluate the impact of educative summer camp on the glycemic control in two different age-groups of young diabetic patients. METHODS: 54 patients (36 M, 18 F, age 10-27 years, duration of diabetes 2-19 years), treated with 0.81 +/- 0.2 UI/kg/day of insulin and with HbA1c mean levels of 8.25 +/- 1.35 g% were followed by an equip of 8 medical doctors, 4 nurses and 1 dietician for a week during an educative summer camp. RESULTS: 34 children, group 1 (20 M, 14 F, 10-14 years aged, mean duration of the disease 4.52 years, range 2-12 years) and 20 young adults, group 2 (16 M, 4 F, age 16-27 years, mean duration 10.21 years, range 2-19 years) were evaluated. Insulin doses and HbA1c levels were 0.82 +/- 0.21 UI/kg/day vs 0.80 +/- 0.22 U/kg/day and 9.54 +/- 1.5% vs 7.6 +/- 0.6%, p < 0.02 in group 1 and 2 respectively. Glycemic levels at 8 a.m. and 11 p.m. were significantly higher in group 1 than in group 2 (180 +/- 87 mg% vs 219 +/- 77 mg%, p < 0.05 and 164 +/- 84 mg% vs 201 +/- 81 mg%, p < 0.05). Hypoglycemic/patient/episodes were 1.82 vs 0.72, p < 0.05 in group 1 and group 2 respectively. CONCLUSIONS: 1. Glycemic control was unsatisfactory in both groups and it was significantly worse in the group of youngs, though in this group HbA1c level was significantly lower. 2. The risk of hypoglycemia was significantly higher in group 1, though in this group insulin doses were significantly decreased.

Complement-fixing islet cell antibodies in type-1 diabetes can trigger the assembly of the terminal complement complex on human islet cells and are potentially cytotoxic.

Forty-one sera of patients with IDDM (insulin-dependent diabetes mellitus) containing complement-fixing islet cell antibodies were analyzed for their ability to activate TCC (terminal complement complex). Eighteen sera were found to promote deposition of TCC on human islets of pancreatic cryostat sections with a nonhomogeneous pattern of distribution corresponding to that of insulin. Activation of TCC by IDDM serum and binding of this complex to islet cells was confirmed using purified islets. Flow cytometric analysis of islet cell treated with a TCC+ IDDM serum showed IgG binding to the cell surface. The same serum had a cytotoxic effect on islet cells in the presence of human C. These results obtained with a homologous system of C activation by IDDM sera suggest that TCC may contribute, at least in part, to the pancreatic beta cell damage.

[Evaluation of a rapid and simple ambulatory method for the determination of HbA1c in diabetic children]. / Valutazione di una metodica ambulatoriale rapida e di semplice esecuzione per la determinazione dell'HbA1c nei bambini diabetici.

The aim of this study was to compare a simply and quick outpatient method for the determination of HbA1c (DCA 2000 Bayer) to a laboratory immunoturbidimetric method (B M Hitachi 911). We have determined HbA1c in 139 subjects (120 diabetics and 19 healthy controls, 81 males and 58 females, mean age ( +/- DS) 14.4 +/- 6.41 years, range 2-40) with the two methods at the same time. The HbA1c normal range, determined by DCA 2000 and by laboratory method, were 4-5.2% and 4.5-6% respectively. The HbA1c values determined with both methods were significantly correlated (r = 0.91; p < 0.001). In 16 diabetic patients, chosen for their reliability, the HbA1c values determined by DCA 2000 were significantly correlated to the last two month mean glycemic level preceding the test. In 14 healthy subjects HbA1c was determined simultaneously by DCA 2000 in capillary and venous blood samples. The values were similar. The DCA 2000 method showed to be reliable, simply to use, no traumatic and ideal for pediatric outpatient use.

A case of infant botulism associated with honey feeding in Italy.

A case of infant botulism in a 9 week-old female is described. A strain of C. botulinum type B was isolated from the feces of the baby. The epidemiologic study detected in a sample of home canned honey Clostridium botulinum spores of the same serotype that was isolated from the patient. The honey had been used only to sweeten the pacifier of the baby. This is the first case of infant botulism in Europe linked conclusively to honey.

[Distribution of new cases of insulin-dependent diabetes mellitus (IDDM) by age, sex, seasonality, and clinical characteristics at onset in youngsters from the Friuli Venezia Giulia region from 1987 to 1990]. / Distribuzione dei nuovi casi di diabete mellito insulinodipendente (DID) per età, sesso, stagionalità e caratteristiche cliniche all'esordio nei giovani della Regione Friuli Venezia Giulia durante il quadriennio 1987-'90.

A retrospective study was undertaken to estimate the incidence of insulin-dependent diabetes mellitus (IDDM) in youngs (< 18 years) in a North-East Province of Italy: Friuli Venezia Giulia (total population: 1,211,320; under age 18: 185,860). On the assumption that all children with newly detected IDDM are referred to a hospital, data were collected from all pediatric departments (14) and from all diabetologic services (14) of the Province. A central register for all patients receiving drug reimbursement was used as the secondary source for validation of case ascertainment, that was 98%. All IDDM cases diagnosed between 1987 and 1990, with age onset < 18 years, and using insulin at discharge from hospital, were included. 73 new IDDM cases under 18 years (47 M, 26 F) were observed during the considered period. The average annual incidence of the disease (based on first hospital admission) was estimated to be 9.8 per 100,000 children under 18 years. There was a significantly higher incidence for boys (12.6/100,000) than for girls (6.9/100,000). The highest incidence (12.5/100,000) was in the age group 10-15, and the lowest (7.2/100,000) in the aged 0-5 years. First admission rates showed seasonal variations, with peaks in Oct/Nov and Jan/Feb. History of an infection during the month before the first admission was present in 17/73 cases. Duration of symptoms < 15 days, 15-30 days, > 30 days, was present in 43, 18, 12 patients respectively. Weight loss < 5% was present in 31/73 cases. Ketoacidosis at onset (pH < 7.36) was present in 30/73 cases.(ABSTRACT TRUNCATED AT 250 WORDS)

[Comparison of high-dose immunoglobulin and cyclosporine in newly diagnosed diabetic children]. / Confronto fra immunoglobuline ad alte dosi e ciclosporina in bambini diabetici all'esordio.

A controlled trial was carried out on type I diabetic children to evaluate and to compare the clinical effects of two different kinds of immunotherapy: high doses intravenous gammaglobulin (IVIgG) and cyclosporine A (CyA). 30 newly diagnosed patients were admitted to the trial, 10 of whom served as controls (group A), 10 received 400 mg/kg b.w. of IVIgG on 5 consecutive alternate days and subsequently after 15 days and monthly thereafter for up to six months (group B), 10 patients received CyA 5-10/kg b.w. by mouth in two daily doses for a period comprised between 6 and 18 months (group C). Serum post-prandial C-peptide level was significantly higher after 6 months in group B and C than in group A; after 12 months, only group C showed significantly higher values. This difference was no longer significative at 18 and 24 months. Insulin requirement in the treated groups was significantly lower than in control group at 6 months, this difference was no longer significative at 12 months. We didn't find any difference concerning insulin requirement during the study comparing the two groups treated with the two different immunosuppressive therapies. In 3 patients in group B and in 3 patients in group C we didn't observe any appreciable response to immunosuppressive therapy (defined as insulin requirement greater than 0.5 UI/kg b.w. at 6 months and/or greater than 0.8 UI/kg b.w. at 12 months). We couldn't find any significant difference between responders and not responders to the immunosuppressive therapies regarding age, symptoms lasting before the diagnosis, weight loss, ketoacidosis intensity and serum post-prandial C-peptide level at the onset.(ABSTRACT TRUNCATED AT 250 WORDS)

Screening of diabetic children for coeliac disease with antigliadin antibodies and HLA typing.

IgA and IgG antigliadin antibodies were measured in 498 patients with insulin dependent diabetes mellitus and no history of intestinal malabsorption. Thirty patients had abnormal concentrations of antigliadin antibodies; 22 of these had an intestinal biopsy carried out and 16 of the 22 had subtotal villous atrophy suggestive of coeliac disease (prevalence 3.2%). There were no significant differences between patients with coeliac disease and diabetes and diabetic patients with normal IgA antigliadin antibodies in any of the nutritional variables measured, duration of diabetes, and mean insulin requirement. The mean age of onset of diabetes and attainment of expected height for age were both significantly lower in the patients with both diseases. Typing HLA classes I and II was done in 242 patients. The incidence of HLA-B8, DR3, and DQW2, which are commonly associated with both the diseases, is increased when both are present.